Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt–Jakob Disease: A Retrospective Study in 215 Autopsy Cases

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023884%3A_____%2F22%3A00009392" target="_blank" >RIV/00023884:_____/22:00009392 - isvavai.cz</a>

Alternative codes found

RIV/00064173:_____/22:43923227 RIV/00064165:_____/22:10442399 RIV/00216208:11110/22:10442399 RIV/00216208:11120/22:43923227 and 2 more

Result on the web

<a href="https://www.mdpi.com/2227-9059/10/3/680" target="_blank" >https://www.mdpi.com/2227-9059/10/3/680</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.3390/biomedicines10030680" target="_blank" >10.3390/biomedicines10030680</a>

Result language

angličtina

Original language name

Biomarkers Analysis and Clinical Manifestations in Comorbid Creutzfeldt–Jakob Disease: A Retrospective Study in 215 Autopsy Cases

Original language description

Creutzfeldt-Jakob disease (CJD), the most common human prion disorder, may occur as "pure" neurodegeneration with isolated prion deposits in the brain tissue; however, comorbid cases with different concomitant neurodegenerative diseases have been reported. This retrospective study examined correlations of clinical, neuropathological, molecular-genetic, immunological, and neuroimaging biomarkers in pure and comorbid CJD. A total of 215 patients have been diagnosed with CJD during the last ten years by the Czech National Center for Prion Disorder Surveillance. Data were collected from all patients with respect to diagnostic criteria for probable CJD, including clinical description, EEG, MRI, and CSF findings. A detailed neuropathological analysis uncovered that only 11.16% were "pure" CJD, while 62.79% had comorbid tauopathy, 20.47% had Alzheimer's disease, 3.26% had frontotemporal lobar degeneration, and 2.33% had synucleinopathy. The comorbid subgroup analysis revealed that tauopathy was linked to putaminal hyperintensity on MRIs, and AD mainly impacted the age of onset, hippocampal atrophy on MRIs, and beta-amyloid levels in the CSF. The retrospective data analysis found a surprisingly high proportion of comorbid neuropathologies; only 11% of cases were verified as "pure" CJD, i.e., lacking hallmarks of other neurodegenerations. Comorbid neuropathologies can impact disease manifestation and can complicate the clinical diagnosis of CJD.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30210 - Clinical neurology

Project

<a href="/en/project/NV19-04-00090" target="_blank" >NV19-04-00090: Overlap of neurodegenerative dementias and their clinic-pathological correlations: a prospective-retrospective multicentric study</a><br>

Continuities

N - Vyzkumna aktivita podporovana z neverejnych zdroju

Publication year

2022

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Biomedicines

ISSN

2227-9059

e-ISSN

—

Volume of the periodical

10

Issue of the periodical within the volume

3

Country of publishing house

CH - SWITZERLAND

Number of pages

14

Pages from-to

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UT code for WoS article

000775872000001

EID of the result in the Scopus database

2-s2.0-85129860953